CASE REPORTS

Splenogonadal fusion: report of a rare variety

Case report

Comment References

We report an unusual case of splenogonal fusion in a 10-year-old boy with an undescended left testis. He suffered from congenital limb defects, a known association with splenogonadal fusion, and had originally been admitted for orchidopexy.

Keywords: Splenogonadal fusion, technetium 99 scan

J.R.Coll.Surg.Edinb., 46, April 2001, 108-109 

CASE REPORT

A 10-year-old boy with an undescended left testis was admitted for orchidopexy. He suffered from congenital limb defects, a known association with splenogonadal fusion.^1-5

Ultrasound revealed a testis in the left groin and normal kidneys and spleen with no features of splenogonadal fusion. Formal groin exploration revealed a testis with an abnormal single arterial connection passing from the testis via the deep ring transperitoneally to the spleen (Figure 1). Unsure of the nature of the testicular blood supply, the vascular connection was preserved and a standard orchidopexy performed. Following surgery, the boy recovered well with the left testis in the left scrotum. In view of the possibility of aberrant splenic tissue within the vascular connection, a denatured red cell scan using technetium 99 was performed postoperatively. This demonstrated functioning splenic tissue throughout the vascular connection (Figure 2). A repeat ultrasound in light of the above information was once again normal.

Figure 1: Intraoperative view shows abnormal single arterial connection between left testis and spleen

Figure 2: Denatured red cell scan using Technetium 99 showing functioning splenic tissue throughout the vascular connection.

COMMENT

Cryptorchidism is the commonest anomaly associated with splenogonadal fusion. Other associated anomalies include bilateral absent legs, imperforate anus, spina bifida, diaphragmatic hernia and hypospadias.^1-5 Preoperative diagnosis of this rare condition is difficult. Ultrasound⁶ and technetium-99m-sulfur colloid scan² have been suggested. Putschar and Manion classified splenogonadal fusion into two types. A continuous type, in which the principal spleen is connected to the gonad by a cord like structure that may be fibrous, totally splenic or beaded with multiple splenic nodules, and a discontinuous type, in which there is no connection between the main spleen and the gonad.⁷ Our case appears to be unique as the communication was vascular beaded with splenic tissues. This case illustrates the unreliability of ultrasound and the value of technetium 99 scan to detect splenogonadal fusion.

REFERENCES

1. Cortes D, Thorup JM and Visfeld J. The pathogenesis of cryptorchidism and splenogonadal fusion: a new hypothesis. Brit J Urol 1996; 77: 285-90
2. Guarin U, Dimitrieva Z, Ashley SJ. Splenogonadal fusion - a rare congenital anomaly demonstrated by 99mTc-sulfur colloid imaging: case report. J Nucl Med 1975; 16: 922-4
3. Carragher AM. One hundred years of splenogonadal fusion. Urology 1990; 35: 471-5
4. Lipson AH. Amelia of the arms and femur/fibula deficiency withsplenogonadal fusion in a child born to a consanguineous couple. Am J Med Genet 1995; 55: 265-8
5. Gouw ASH, Elema JD, Bink-Boelkens MTE, de Jongh HJ, ten Kate LP. The spectrum of splenogonadal fusion: case report and review of 84 reported cases. Eur J Pediatr 1985;144:316-23
6. Patel RV. Splenogonadal fusion. J Pediatr Surg 1995; 30: 873-4
7. Putschar WGJ, Manion WC. Splenic-gonadal fusion. Am J Pathol 1956; 32: 15-35

Copyright date: 12th June 2000

Correspondence: Mr ME Foster, Consultant Surgeon, Surgical Department, Royal Glamorgan Hospital, Ynysmaerdy, Llantrisant CF72 8XR, U.K.

©2001 The Royal College of Surgeons of Edinburgh, J.R.Coll.Surg.Edinb.